ONCOLOGY LETTERS 7: 728-730, 2014
Small cell neuroendocrine carcinoma of the ureter: A case report and literature review JIANG HAI PING1, ZHANG XIAO CHEN1, QIAN JIONG1, YOU QI HAN2 and XU NONG1 Departments of 1Medical Oncology and 2Pathology, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China Received May 21, 2013; Accepted November 25, 2013 DOI: 10.3892/ol.2013.1757 Abstract. Small cell neuroendocrine carcinoma arising in the ureter is extremely rare; only a few cases have been previously reported in the literature. The current study reports the case of a 65‑year‑old female who presented with right‑sided back pain. A mass was identified in the right ureter, and a nephroureterectomy was performed. The microscopic examination revealed that the mass was composed of a monotonous population of small cells and that the cells of the carcinoma were positive for cluster of differentiation 56, chromogranin A and synaptophysin. The tumor was diagnosed as a ureteral neuroendocrine small cell carcinoma. The patient returned 4 months later with recurrences in the retroperitoneum. Chemotherapy was administered and following 80 mg/m2 intravenous irinotecan on days 1 and 8 and 25 mg/m2 cisplatin on days 1‑3, every 21 days for 4 cycles, the tumor was considerably smaller. During the regular follow‑up examinations, the tumor remained stable. Introduction Neuroendocrine carcinomas consists of a heterogeneous group of neoplasms, which arise from peptide‑ or amine‑producing endocrine cells throughout the body (1). The most frequent primary locations of the tumors are pulmonary. The extrapulmonary counterpart is rarely encountered, and has been identified in the gastrointestinal tract, pancreas, genitourinary tract, lymphatics, thymus and peritoneum (2). In the genitourinary tract, the bladder is the most common primary site of the disease, while tumors originating from the ureter are extremely rare (3). Due to its rarity, little is known concerning the natural history of ureteral small cell neuroendocrine carcinoma. The current report presents a case and systematic literature review on the clinical presentation and treatment of this rare tumor.
Correspondence to: Dr Xu Nong, Department of Medical Oncology, The First Affiliated Hospital, School of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, Zhejiang 310003, P.R. China E‑mail: [email protected]
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Key words: ureter, neuroendocrine carcinoma, chemotherapy
Case report A 65‑year‑old female presented with right‑sided back pain that had been present for one month. The patient had no significant past medical or family history of disease. The physical examination was normal, and laboratory test results showed normal liver function, electrolytes, carcinoembryonic antigen and carbohydrate antigen 19‑9. The patient provided written informed consent. A computed tomography (CT) scan of the abdomen revealed a mass measuring ~5x4 cm in the right ureter, with light hydronephrosis (Fig. 1). A chest CT was also reviewed and no primary or metastatic lung lesions were revealed. The patient underwent a right nephroureterectomy and a mass was found within the wall of the right ureter, with grossly negative surgical margins. The microscopic examination showed that the tumor was composed of small cells (Fig. 2). The immunohistochemical staining for the tumor cells was positive for cluster of differentiation (CD)56 (Fig. 3A), chromogranin A (CgA; Fig. 3B) and synaptophysin (Syn; Fig. 3C). Moreover, the tumor expressed high mitotic activity of >20 mitoses per 2 mm 2, and the Ki‑67/MiBi index was 67% (Fig. 3D). The patient was diagnosed with small cell neuroendocrine carcinoma of the ureter. The post‑operative recovery of the patient was uncomplicated, but the patient returned 4 months later, with CT scans revealing recurrences in the retroperitoneum (Fig. 4A). Chemotherapy was administered and following 80 mg/m 2 intravenous irinotecan on days 1 and 8 and 25 mg/m 2 cisplatin on days 1‑3, every 21 days for 4 cycles, CT scans showed a considerably smaller tumor (Fig. 4B). During the regular follow‑up examinations, the tumor remained stable (Fig. 4C). Discussion Neuroendocrine carcinomas arising from the urinary tract are extremely rare and represent